IRANIAN JOURNAL OF PEDIATRIC HEMATOLOGY AND ONCOLOGY
Year:2018 | Volume:8 | Issue:3|Papers Count:8

Background: Neuroendocrine Neoplasms (NEN) represent 60% of all appendicular tumours. This type of cancer is predominantly benign. In this study, appendicular NEN tumours in children were investigated. Materials and Methods: This retrospective study was conducted on 540 patients underwent emergency appendectomy for the treatment of clinically suspected appendicitis at the department of pediatric surgery in Hedi Chaker hospital in Sfax, Tunisia. This study was performed between January 2013 and December 2016. Data on appendicular NEN demographic, preoperative diagnoses, investigations, operative findings, pathological reports, and follow up were reviewed and analysed. Results: Out of 540 patients, there were 309 males (57. 3%) and 231 females (42. 7%). The Mean age of the patients was 9. 23 ± 2. 78 years. One hundred and thirty seven patients (25. 4%) had laparoscopic appendectomy and 74. 6% were operated by the traditional open approach. The diagnosis of appendicular NEN tumours was histologically confirmed in 4 appendectomy specimens. Clinically, all patients presented with acute appendicitis with raised inflammatory markers and positive ultrasound for appendicitis. The mean of tumour size was 1 cm. Complete resection was successfully achieved in all patients. The mean of follow-up was 3 years. Conclusion: Appendicular NEN tumours were diagnosed postoperatively with a histological examination. It seems that surgical management with simple appendectomy seems to be curative for the majority of cases especially with tumours less than 2 cm. according to this study, the prevalence of appendicular NEN tumours is low between all gastrointestinal tumors.

Background: The brain and spinal cord tumors account for 15% to 20% of all childhood malignancies. It is important to know the epidemiologic characteristics and survival of these patients to better understand the disease and the factors affecting its prognosis. The aim pf this study was to characterize the clinicopathology and survival rate of childhood and adolescent brain and spinal cord tumors in center of Iran. Materials and methods: This descriptive-analytic study was carried out using a retrospective cohort design. Thirty patients with brain and spinal cord tumors who referred to Shahid Sadoughi and Rahnemoon hospitals in Yazd from 2006 to 2016 and aged 1 to 18 years were evaluated. . The epidemiologic characteristics, survival, and the factors affecting the survival of brain and spinal cord tumors were investigated. Results: The findings showed that between 30 studied patients, brain and spinal cord tumors were more common in males (19 males and 11 females). The average age of the patients was 8. 60 ± 5. 70 years. Fifteen (50%) patients survived. Seventeen (57%) patients were resident in Yazd province and 13 (43%) were from southern Iran. Twenty two patients (73. 3%) had recurrence after recovery. The average of survival was 36 months, with an average of 27 months in females and 37 months in males. However, this difference was not significant. The most common tumor was gliomas. There was no significant relationship between the mean of survival with age, gender, geographical status, or type of treatment (P value> 0. 05); however, there was a significant relationship between the year of tumor diagnosis and survival (P value=0. 0134). Conclusion: It seems that survival of the brain and spinal cord tumors in children and adolescence is a multifactor event and it is affected by various factors.

Background: Niosomes or Nonionic surfactant vesicles are nano vehicles utilized in drug delivery systems, especially in cancer therapy. In this study, these vesicles were applied as delivery system for anticancer drug, paclitaxel and then, its anticancer activities was compared with free paclitaxel on Human Acute Lymphoblastic Leukemia (ALL) cell line Nalm-6. Materialas and Methods: In this exprimental study, paclitaxel loaded niosome was prepared by thin film hydration method. The characterization tests included dynamic light scattering (DLS) and UV-Vis spectrophotometry were employed to evaluate the quality of the nanocarriers. Cytotoxicity of niosomal paclitaxel nanoparticles and free paclitaxel on human acute lymphoblastic leukemia cell line Nalm-6 after 24 hours were studied by MTT assay to determine cell viability. Results: Percent of encapsulation paclitaxel prepared with sorbitane monostearate, cholesterol, and DSPEmPEG 2000 was 97. 21 %. In addition, the polydispersity index, mean size diameter, and zeta potentials of niosomal paclitaxel nanoparticles were found to be 0. 244 ± 0. 011, 106. 3 ± 1. 5 nm, and-26. 03 ± 1. 34; respectively. Paclitaxel released from nanoniosome in 72 h was 19. 81 %. The results demonstrated that a 2. 5∼ fold reduction in paclitaxel concentration was measured when the paclitaxel administered in nanoniosome compared to free paclitaxel solution in human acute lymphoblastic leukemia cell line Nalm-6. Conclusion: As a result, the nanoparticle-based formulation of paclitaxel has high potential as an adjuvant therapy for clinical usage in human acute lymphoblastic leukemia therapy.

Background: Children with thalassemia do not have favorable psychological health. Today, the use of different therapeutic regimes for caregivers with thalassemia has increased life expectancy; however, these patients have other various needs and requirements such as constant professional training. The aim of the present study was to explain stress management in caregivers with thalassemia children. Materials and Methods: The method applied in this study was phenomenological qualitative research with conventional content analysis. This study was done in 2016-2017 in Shahrekord, Iran. A total of 15 participants, including 10 mothers, 1 grandmother, and 4 fathers participated purposely in this study. There were two sessions of interviews each of which lasted for 30 minutes. The content of interview was recorded and analyzed through conventional content analysis method after documentation. Results: Following content analysis, three categories were obtained: seeking for hope included two subcategories of seeking for hope and trusting in God, seeking for information included two subcategories of seeking for information from parents and seeking for information from physician and nurse, and seeking for new treatment included two subcategories of seeking for new treatment and seeking for transplant. Conclusion: This findings of the present study showed that planners and healthcare team can contribute caregivers of thalassemic children in coping with this disease using three adaptation approaches; that is, offering caregivers hope and providing them with proper information about the disease and treatments.

Background: The occurrence of medical errors in therapeutic centers is important due to its critical nature in terms of health, patient safety, and notable clinical and economic outcomes. One of the solutions to manage this problem in the field of nursing is error reporting and recording. Error reporting, on one hand, improves patient care quality and safety and; on the other hand, provides valuable information to prevent future errors. Therefore, considering the importance of error reporting, the aim of this study was to determine the effect of senior managers' compliance in reporting nurses' treatment error in pediatric ward of Shahid Sadoughi Hospital in Yazd. Materials and Methods: This interventional study included all nurses working in pediatric wards. The intervention was defined as various safety management drivers and encouragement of staff to report errors without any fears or concerns from senior managers. The error reports was recorded and comprised before and after intervention. For daat anlysis, SPSS (version 21) was run. Results: Following the intervention, over the course of a year, a total of 327 errors were reported. With respect to wards, 36. 9% of errors occurred in pediatric oncology ward, 40% in PICU, 25. 6% in pediatrics, 8% in emergency department of pediatrics, and 15. 9% in NICU. However, only 32 errors were reported during the last year. Data analysis indicated a significant increase in error reporting following the intervention (P-value = 0. 021). Furthermore, the results showed that mostly errors occurred in morning shift. Considering the error type, medication error was the most frequent; and considering the reason, non-compliment with the principles of drug adminstration got the highest frequency. Conclusion: The most important step in reducing errors is to eliminate the obstacles against reporting errors by creating a situation in which each nursing staff can honestly report his/her error. Therefore, regarding a significant difference before and after the intervention, it is recommended that senior managers consider medical treatment error reporting as their priorities.

Background: Somatic mutations in the hotspot region of the DNA-methyltransferase 3A (DNMT3A) gene were recurrently identified in acute myeloid leukemia (AML). It is believed that DNMT3A mutations confer an adverse prognosis for AML patients. These lines of evidence support the need for a rapid and cost-efficient method for the detection of these mutations. The present study aimed to establish high resolution melting (HRM) curve analysis as a rapid and sensitive test to identify DNMT3A gene mutations in AML patients. Materials and Methods: In this retrospective cohort study, a total of 220 AML patients who referred to hematology-oncology and stem cell transplantation centers (referral center) at Shariati hospital in Tehran, Iran, were included. AML-M3 and therapy-related AML patients were excluded. The HRM assay was used to identify R882 mutations in DNMT3A gene, and the results were compared with those of Sanger sequencing as the gold standard test for detection of such mutations. Results: Among 220 samples from AML patients, Sanger sequencing detected 25 (11. 4%) patients as having DNMT3A R882 mutations. HRM assay detected mutations in 23 (92%) samples and reported two false-negative results that were related to poor-quality DNA samples. There was an overall good agreement between direct sequencing and HRM assay (kappa value of 0. 95) (p<0. 001). Sensitivity assay showed that the analytical detection limits for HRM were 10% for the detection of R882H mutation compared with Sanger sequencing at 25%. Both Sanger sequencing and HRM assay reported no false-positive results. Conclusion: HRM curve analysis can be considered as a sensitive, fast, and high-throughput method for the detection of DNMT3A R882 mutation in AML patients. These results validate HRM analysis as an alternative method to Sanger sequencing because of its simplicity along with the lower cost and less required time.

Vitamin D deficiency is known as the most common nutritional deficiency. It is created during infancy due to different factors, including decreased dietary intake, decreased dermal synthesis, malabsorption, enzymeinducing medications, and exclusive breastfeeding. Vitamin D deficiency is associated with poor bone health such as rickets and osteomalacia in children. Despite vitamin D plays an important role in bone health, its role in pediatric cancer is not detected and remained unknown; therefore, the aim of this study was to evaluate the role of vitamin D deficiency and its relation with cancer in children. Vitamin D in cancer children has been considered as a contributory factor for skeletal pathologies. Children with cancer may be at increased risk of vitamin D deficiency due to side effects which are induced by the disease and multiple treatments, given that chemotherapy and clinical radiation play a main role in decreased bone mineral density. Therefore, possible role of vitamin D deficiency in cancer pathogenesis and progression is well defined. It seems that these patients should be taken sufficient amount of calcium and vitamin D during chemotherapy and afterward.

Background: Para-amino benzoic acid (PABA) is an important ingredient used as a structure moiety in drugs with wide range of therapeutic uses; however, its safety and possible side effects in young children have not been determined. Case Presentation: An 8-year-old girl was admitted to the emergency department for paleness, jaundice, abdominal pain and nausea associated with Hemoglubin (Hb) =4. 5 g/dl, reticulocytes = 6%, corrected reticulocytes = 1. 8%, Lactate Dehydrogenase (LDH) = 1893 IU/L, and Total bilirubin =12 mg/dl with direct bilirubin = 0. 7 mg/dl. The patient had received a prescription for PABA, in order to fade out some facial hypopigmented macules, for 120 days prior to her admission. Within 120 days of starting the PABA, the patient had developed new onset abdominal pain following each meal, weight gain, paleness, and jaundice. The PABA was discontinued on the day of admission. Hematologic evaluation revealed no evidence of autoimmune hemolytic anemia, glucose 6 phosphate dehydrogenase (G6PD) deficiency, red blood cell (RBC) membrane defects, or hemoglubinopathy. Moreover, hepatologic evaluation revealed no evidence of acute or chronic viral hepatitis, autoimmune or metabolic disorders at admission. During the admission, her transaminase and gama glutamil transpeptidase (GGT) levels increased more than 5 times without any elevation in international normalized ratio (INR) or alkaline phosphatase. Moreover, abdominal ultrasonography revealed acalculous cholecystitis. Her Hb, Hct (Hematocrit), reticulocytes, liver enzymes, bilirubin, and gallbladder ultrasonography completely normalized 2 months after discontinuation of PABA. Conclusion: This report represented the first documented case of hemolytic anemia and hepatotoxicity in a child underwent PABA therapy, highlighting the need for clinician awareness about potential hemolytic anemia and hepatotoxicity of oral PABA particularly among children.